Fig. 44.1
A clinical photograph showing a large congenital pancreatic cyst
They may however be symptomatic as a result of pressure on adjacent structures leading to:
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Abdominal distention
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Vomiting
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Jaundice
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Pancreatitis
The majority of patients present before the age of 2 years, and associated anomalies were found in 30% of cases. These include:
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Asphyxiating thoracic dysplasia (Jeune syndrome)
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Short-limb dwarfism
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Polydactyly
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von Hippel–Lindau disease
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Beckwith–Wiedemann syndrome
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Hemihypertrophy
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Renal tubular ectasia
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Anorectal malformation
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Polycystic kidneys
Diagnosis
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Modern imaging techniques usually show a well-defined unilocular cyst; however, even with the combined use of laboratory data, clinical features, and diagnostic imaging, it may be difficult to accurately differentiate congenital pancreatic cyst from other nearby cystic lesions of the abdomen. This is specially so if they are large in size (Figs. 44.2, 44.3, 44.4, and 44.5).Fig. 44.2a and b Abdominal ultrasound showing a large pancreatic congenital cystFig. 44.3Abdominal CT scan showing a very large congenital pancreatic cyst. Note the thick wall of the cyst
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